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ID PMID Title PublicationDate abstract
21194883 Pulmonary manifestations of Sjögren's syndrome. 2011 Jan Sjögren's syndrome is a chronic inflammatory disorder characterized by lymphocytic infiltration of exocrine glands, mainly the lacrimal and salivary glands. However, extraglandular organ systems may frequently be involved, including the lungs. Although subclinical pulmonary inflammation exists in more than 50% of patients, clinically significant pulmonary involvement affects approximately 10% of patients and may be the first manifestation of the disease. The entire respiratory tract may be involved, with a wide spectrum of manifestations including xerotrachea and bronchial sicca, obstructive small airway disease, various patterns of interstitial lung disease, lymphoinfiltrative or lymphoproliferative lung disease, such as lymphoma (usually of MALT type), pulmonary hypertension, pleural involvement, lung cysts, and pulmonary amyloidosis.
21069527 Diagnosis and management of lymphoepithelial lesion of the parotid gland. 2011 Jul Patients with either benign or malignant parotid neoplasm are candidates for surgery, but patients with benign lymphoepithelial lesions of the parotid gland or Sjögren's syndrome do not necessarily require surgical treatment. However, the diagnosis of benign lymphoepithelial lesion of the parotid prior to surgery is challenging. In this case series, we retrospectively analyzed the records of 11 patients presented between January 2006 and August 2007, with a solitary parotid mass diagnosed post-operatively as benign lymphoepithelial lesion or Sjögren's syndrome. Our analysis suggested that findings from physical examination and CT and MRI scans in the absence of neoplastic cells on fine needle aspiration biopsy could be used to make the diagnosis of lymphoepithelial lesion preoperatively. In a prospective study from September 2007 to June 2008, using the lessons learned from the analysis of the previous 11 patients, we were able to diagnose all 6 cases of benign lymphoepithelial lesion or Sjögren's syndrome preoperatively.
20810502 Impaired gastric emptying in primary Sjogren's syndrome. 2010 Nov OBJECTIVE: To investigate the prevalence of impaired gastric emptying (IGE) and its relation to autonomic nervous dysfunction (AD), functional bowel syndrome, and inflammatory and serological variables in patients with primary Sjögren's syndrome (pSS). METHODS: Twenty-eight patients with pSS according to the American-European Consensus Criteria were included in the study. Gastric emptying was evaluated by the octanoate breath test from which half-time (t(half)) and lag-time (t(lag)) were determined and compared with the results from 50 healthy controls. Autonomic nervous function was evaluated by 5 objective autonomic reflex tests (ART) and by the Autonomic Symptom Profile (ASP) questionnaire evaluating AD symptoms. These results were compared with previously investigated healthy ART controls and population-based ASP controls. Patients were also assessed regarding symptoms of functional bowel syndrome. RESULTS: The t(half) and the t(lag) were significantly prolonged in patients compared to controls. Forty-three percent of patients with pSS presented signs of IGE and 29% fulfilled the criteria for gastroparesis. Significant correlations were found between t(lag) and increased levels of IgG (p = 0.02) and erythrocyte sedimentation rate (ESR; p = 0.01). In addition, rheumatoid factor (RF) seropositives showed objective signs of IGE to a greater extent than RF seronegatives. No associations between IGE, ART variables, ASP variables, or gastrointestinal symptoms were found. CONCLUSION: IGE was common in pSS. Associations with inflammatory and serological features of pSS could imply immunological mechanisms behind the IGE. Objective signs of IGE were not associated with objective signs or subjective symptoms of AD or functional bowel syndrome.
20362362 [McDuffie hypocomplementemic urticarial vasculitis associated with Sjögren's syndrome]. 2010 Jul We report a patient with Sjögren's syndrome who presented with urticarial hypocomplementemic vasculitis. A 46-year-old female was admitted for assessment of ascitis. Clinical examination and computed tomographic scan disclosed evidence of multiple peripheral and intra abdominal lymph nodes. During her admission, she developed several bouts of acute angioedema and urticarial skin lesions. Minor salivary gland biopsy showed focal sialadenitis, stage IV of Chisholm. Schirmer's test was positive. Laboratory examination found low levels of C1q and high levels of C1q antibodies. Therapy with prednisone and hydroxychloroquine was initiated. Six months later, the patient presented with lower limb oedema. Urinalysis showed proteinuria (1g/day) and renal biopsy revealed membranous nephropathy with favorable outcome with corticosteroids.
19955202 In vivo confocal microscopy of conjunctival goblet cells in patients with Sjogren's syndro 2010 Nov BACKGROUND: To study the morphology and the density of conjunctival goblet cells (GC) in patients with Sjögren's syndrome dry eye with in vivo laser scanning confocal microscopy (LSCM), and to explore its correlation with the GC density detected by impression cytology. METHODS: A total of 43 Sjögren's syndrome dry eye patients were recruited. All were required to fill in the Ocular Surface Disease Index Questionnaires. The tear break-up time was measured, followed by corneal fluorescein staining examination and Schirmer I test. The images of conjunctiva were taken by the Heidelberg retina tomography (HRT-II)/Rostock cornea module. Finally, the specimens for impression cytology were obtained. SPSS V.13.0 software was used to analyse the data. RESULTS: Tear film function test showed that all patients had moderate to severe dry eye. The GC in LSCM images was characterised as a large hyper-reflective oval-shape cell with relatively homogeneous brightness. Though GC density assessed by LSCM (332 ± 137) cells/mm² was higher than that measured by impression cytology (200 ± 141) cells/mm², they showed a significant positive correlation, ρ = 0.908 (p < 0.05). Conclusion Conjunctival GC could be easily discriminated under the LSCM. LSCM may be a valuable tool in monitoring the progress and the follow-up of patients with Sjögren's syndrome dry eye.
20498211 Salivary gland ultrasonography: can it be an alternative to sialography as an imaging moda 2010 Jul OBJECTIVES: Salivary ultrasonography (US) was evaluated as an alternative imaging modality to sialography for Sjögren's syndrome (SS). METHODS: Parotid sialography and parotid and submandibular US were performed in 360 patients (188 with SS and 172 non-SS) who were suspected of SS and had fulfilled the American-European criteria (AEC) for SS or had received > or =3 of the objective examinations that were considered minimal requirements for classifying non-SS and had undergone both the imaging examinations. The glands were considered positive for SS if they exhibited peripheral sialoectatic changes on sialography and/or hypoechoic areas, echogenic streaks and/or irregular gland margins on US. The images obtained were independently rated as SS-positive or SS-negative by three radiologists in a blind fashion and the final decision was made by consensus. Interobserver and intermodality agreement was evaluated using kappa values for sialography and parotid and/or submandibular US. RESULTS: Average kappa values for the interobserver agreement were 0.81, 0.80 and 0.82 in sialography, parotid and submandibular US, respectively, indicating very good or good agreement. The kappa value for intermodality agreement between sialography and parotid US was 0.81 and between sialography and submandibular US was 0.76, indicating very good and good agreement, respectively. The diagnostic ability of parotid US was significantly lower than that of sialography (p<0.001, McNemar test). However, the diagnostic ability of submandibular US was comparable to that of sialography (p=0.153). CONCLUSIONS: Submandibular US is a promising technique that can be used as a practical alternative to sialography in the classification of SS.
19543861 [A 39-year-old patient with maculopapular rash, recurrent fever, and arthralgia]. 2009 Jul Adult-onset Still's disease is a rare disorder of unknown etiology. We report the case of a 39-year-old patient who showed the characteristic symptoms: recurrent attacks of fever, arthralgia, maculopapular rash, sore throat, and lymphadenopathy. After the possibility of an infectious or paraneoplastic process was excluded and the laboratory findings were evaluated (increased C-reactive protein, liver values, and ferritin level), the diagnosis was established according to the criteria of Yamaguchi. Therapy with steroids and nonsteroidal anti-inflammatory drugs was started successfully.
20934259 Detection of minor salivary glands affected in Sjogren's syndrome by Raman spectroscopy. 2011 Feb Sjogren's syndrome(SS) is defined as a slowly processing autoimmune inflammatory exocrinopathy affecting the salivary and lachrymal glands. Due to the difficulty in reaching a diagnosis in some SS cases, there are many criteria published for classification and diagnosis. Among the most of criteria, lip biopsy plays an important role in clinical diagnosis, but it is an invasive and complex method which always causes patients' pain and discomfort. Raman spectroscopy is a noninvasive and real-time vibrational spectroscopic technique applied in the clinical detection and diagnosis of diseases. The diagnostic sensitivity and specificity are both high. The hypothesis is to detect the minor salivary glands affected in SS by the noninvasive Raman spectroscopy instead of lip biopsy. Therefore, Raman spectroscopy may increase the diagnostic sensitivity and specificity, meanwhile prevent patients from pain caused by biopsy.
20423219 Discrepancy between clinical course and drug-induced lymphocyte stimulation tests in a cas 2010 Apr BACKGROUND: Saireito consists of components of shosaikoto and goreisan. There are several reports of liver injury caused by shosaikoto and saireito, whereas cases caused by goreisan are rarely seen. PATIENT: A 70-year-old woman suffered from sicca of the eyes and oral cavity that arose in 2003. In June 2004, we diagnosed her as having Sjögren syndrome on the basis of Schirmer's test, salivary scintigraphy, presence of anti-SS-A/Ro antibody, and subjective symptoms. Although a muscarinic agonist was commenced, her sicca symptoms did not improve. INTERVENTIONS: In February 2008, 7.5 g/day of shosaikoto extract granules (for ethical use, Tsumura & Co., Tokyo, Japan) was introduced. Her symptoms and laboratory data did not change. The next month, the treatment was replaced by 9.0 g/day of saireito extract granules (for ethical use, Tsumura & Co., Tokyo, Japan). RESULTS: After 10 weeks, elevation of liver enzymes was observed. On suspicion of drug-induced liver injury (DILI), saireito was withdrawn, and the patient's liver enzymes returned to normal the next month. Drug-induced lymphocyte stimulation tests (DLSTs) for saireito, shosaikoto, and goreisan were all positive. CONCLUSIONS: The clinical course suggested allergy to the goreisan component of saireito. It is important to be aware of the limitation of DLST as a diagnostic tool for DILI, especially for herbal medicines.
20236956 Subclinical synovitis in primary Sjögren's syndrome: an ultrasonographic study. 2010 Jun OBJECTIVES: To evaluate, by musculoskeletal ultrasound (MSUS), articular involvement in primary SS (pSS) patients by analysing hand and wrist changes, and to correlate them with clinical evaluation and laboratory tests. METHODS: Thirty-two pSS patients underwent clinical and laboratory examinations, including the SS Disease Damage Index (SSDDI) and the SS Disease Activity Index (SSDAI). MSUS was performed in all patients in both hands and wrists, evaluating the presence of inflammation within joints and periarticular tissues, and the existence of permanent joint damage. For synovial hypertrophy, joint effusion and Doppler signal findings, a semi-quantitative score (0-3) was used indicating the degree of involvement (0 = normal; 1 = mild change; 2 = moderate change; and 3 = severe change). For tenosynovitis and bone erosions, a dichotomous score (0 = absent and 1 = present) was applied. RESULTS: Sonographic signs of synovitis of the radio-ulno-carpal joint were found in 17 (26.5%) out of 64 wrists. Wrist synovitis was found in 12 (37.5%) out of 32 patients. Ultrasonographic examination of the hand did not show significant changes. A statistically significant correlation was found between SSDDI score and the degree of sonographic signs of synovial proliferation in the wrist (P = 0.04). The correlation between the incidence of clinical involvement and the presence of pathological ultrasonographic findings was not significant. Patients with synovitis had a higher median age and higher median SSDDI (P = 0.004). CONCLUSIONS: In pSS patients, MSUS may be considered a useful tool for detecting synovitis since articular involvement can often be silent but correlated with SSDDI.
19790059 Association of immunoglobulin-like transcript 6 deficiency with Sjögren's syndrome. 2009 Oct OBJECTIVE: The immunoglobulin-like transcript (ILT) family is located in chromosomal region 19q13 and consists of a group of activating and inhibitory receptors. The ILT receptors are expressed on antigen-presenting cells (macrophages, dendritic cells, B lymphocytes), as well as on T cells and natural killer cells. ILT2 and ILT4 play a role in tolerance induction, and ILT3 has been shown to induce Treg cells. A homozygous deletion of ILT6 has been shown to be associated with multiple sclerosis. Since ILT6 may be a modulator of the immune system, we undertook this study to examine the association of ILT6 deficiency with Sjögren's syndrome (SS). METHODS: We genotyped 149 patients with SS and 749 healthy controls, using polymerase chain reaction to test for the presence or absence of ILT6. RESULTS: Homozygous ILT6 deficiency was detected in 8% of SS patients and in only 3% of controls. CONCLUSION: Our findings indicate that ILT6 deficiency may be a genetic risk factor for SS.
19947245 [24-year old male with fever, multi-organ dysfunction and fast progressing ARDS]. 2009 Oct A 24-year-old man presented with cough, sore throat, fever, maculopapulous exanthema, pericardial and pleural effusion. Despite extensive evaluation neither infectious, autoimmune, hematological nor oncological disorders were revealed. Broad spectrum antibiotic and subsequently corticosteroid treatment failed to resolve the symptoms. Multiorgan failure with rapid progress of acute respiratory distress syndrome and circulatory failure developed and patient died. Adult onset Still's disease (AOSD), a diagnosis considered in this patient, is a rare disease with unknown prevalence, pathogenesis and etiology. Clinically it is characterized by spiking fever, arthritis, rash, and impairment of multiple organs. There is no single diagnostic test for AOSD. Rather, the diagnosis is based on the clinical criteria and requires the exclusion of infectious, neoplastic, and other autoimmune diseases. Rarely the course of the disease can be rapidly progressive to death. Treatment includes the use of non-steroid antirheumatic drugs and corticosteroids. Limited data suggest that biological agents (e.g. anti-TNF-alpha, anti-IL-1), rituximab or intravenous immunoglobulins might be promising for the treatment of severe cases.
19048255 Fatal myocarditis in adult-onset Still disease with diffuse intravascular coagulation. 2009 Sep Adult-onset Still disease (AOSD) is a rare condition disease of unknown etiology, characterized by quotidian or double quotidian spiking fever, with an evanescent pink-salmon rash, arthritis and multi-organ involvement. Diagnosis is usually clinical and made after other diseases in the differential diagnosis are excluded. We herein report the case of a patient with a remarkable familial autoimmune background in whom adult Still disease started off with a diffuse intravascular coagulation, probably triggered by a macrophage activation syndrome, followed by an acute interstitial myocarditis, leading to a fatal complete atrioventricular block. This case highlights that AOSD represents a troubling condition and that it may suddenly get worse with life-threatening events.
21029481 The effect of immunomodulators on the immunogenicity of TNF-blocking therapeutic monoclona 2010 Therapeutic monoclonal antibodies have revolutionized the treatment of various inflammatory diseases. Immunogenicity against these antibodies has been shown to be clinically important: it is associated with shorter response duration because of diminishing concentrations in the blood and with infusion reactions. Concomitant immunomodulators in the form of methotrexate or azathioprine reduced the immunogenicity of therapeutic antibodies in rheumatoid arthritis, Crohn disease, and juvenile idiopathic arthritis. The occurrence of adverse events does not increase when immunomodulators are added to therapeutic antibodies. The mechanism whereby methotrexate and azathioprine influence immunogenicity remains unclear. Evidence-based consensus on prescribing concomitant immunomodulators is needed.
20452464 Epigenetics in autoimmune disorders: highlights of the 10th Sjögren's syndrome symposium. 2010 Jul During the 10th International Symposium on Sjögren's Syndrome held in Brest, France, from October 1-3, 2009 (http://www.sjogrensymposium-brest2009.org), the creation of an international epigenetic autoimmune group has been proposed to establish gold standards and to launch collaborative studies. During this "epigenetics session", leading experts in the field presented and discussed the most recent developments of this topic in Sjögren's Syndrome research. The "Brest epigenetic task force" was born and has scheduled a meeting in Ljubljana, Slovenia during the 7th Autoimmunity congress in May 2010.The following is a report of that session.
20089471 Influence of swallowing saliva repeatedly on oral moisture. 2010 Jul PURPOSE: The aim of this study was to examine the influence of repetitive saliva-swallowing for the level of oral moisture using an oral-moisture checking device. METHODS: Subjects were 24 healthy adults (HA) and 20 patients with oral dryness, 10 of whom had Sjögren syndrome or who had undergone radiation therapy (ODSR) and 10 who had not experienced ODSR (ODNSR). Oral moisture was measured before swallowing saliva (B), and after swallowing saliva once (F), twice (S), and three times (T). Oral moisture was measured at the lingual mucosa using an oral-moisture checking device. Statistical analysis was performed using the Friedman test. RESULTS: Oral moisture of the lingual mucosa of the HA differed significantly between B and T, and F and T, and the oral moisture level tended to be higher the more times saliva was swallowed. However, oral moisture of lingual mucosa in the ODSR was not different at the different times of measurement, but the level of oral moisture tended to be lower the more times saliva was swallowed. Oral moisture of the lingual mucosa in the ODNSR differed significantly between B and T. The trends for the changes of the oral moisture level in the ODNSR were similar to those in the HA. CONCLUSIONS: The results of this study show that swallowing saliva repeatedly could lead the increase of oral moisture in HA and ODNSR not in ODSR.
20729959 An important difference between "exposed" and "photodistributed" underscores the importanc 2009 Sep This article presents the case of a patient with rheumatoid arthritis who was referred to dermatology for what was suspected to be a photosensitivity reaction to hydroxychloroquine, a common culprit in photodrug reactions. Despite the patient's insistence to the contrary, the cause of her eruption was an airborne allergic contact dermatitis to chemicals she was exposed to during her longtime hobby of soap-making. This case serves as an important illustration of the anatomical differences between eruptions occurring in exposed and photodistributed areas. This article also discusses the causes of dermatitis that may mimic photodrug reactions.
19283906 [The use of statins, a new approach to the treatment of autoimmune diseases]. 2009 HMG-CoA inhibitors (statins) are widely used for the prevention of cardiovascular events and the management of hypercholesterolemia. Recently, multiple cholesterol-independent properties of statins have been discovered. The present review focuses on immunomodulatory and anti-inflammatory effects of statins and their implications for the treatment of autoimmune diseases, such as rheumatoid arthritis, multiple sclerosis, and psoriasis.
20098520 The effect of eszopiclone in patients with insomnia and coexisting rheumatoid arthritis: a 2009 OBJECTIVE: To evaluate the efficacy and safety of eszopiclone 3 mg, a nonbenzodiazepine medication/hypnotic indicated for the treatment of insomnia with comorbid rheumatoid arthritis (RA). METHOD: This multicenter, double-blind, placebo-controlled pilot study was conducted in 153 patients aged 25-64 years with American College of Rheumatology-defined RA who met DSM-IV criteria for insomnia. The data were collected from February to November of 2004. Patients were randomly assigned to either eszopiclone or placebo nightly for 4 weeks, followed by a 2-week placebo run out. Efficacy was evaluated using patient reports of sleep (wake time after sleep onset [WASO], sleep latency [SL], and total sleep time [TST]), daytime function, pain, and RA assessments. Insomnia severity was evaluated using the Insomnia Severity Index. Safety was also evaluated. RESULTS: Eszopiclone significantly improved all patient-reported sleep measures (WASO, SL, and TST), sleep quality, depth of sleep, and daytime function (P < .05 vs placebo). At week 4, 48% of eszopiclone-treated patients had no clinically meaningful insomnia as assessed by ISI score (versus 30% of placebo-treated patients, P = .03). Eszopiclone was significantly better than placebo on some RA-associated pain measures: (1) overall (P = .05), pain (P = .006), and pain and other symptoms (P = .02) scores of the Arthritis Self-Efficacy Scale, (2) tender joint counts (P = .03) and pain severity scores (P = .023), (3) the activities domain of the Health Assessment Questionnaire-Disability Index (P = .04), and (4) the role physical (P = .03) and bodily pain (P = .01) scales of the 36-item Medical Outcomes Study Short-Form General Health Survey. The most commonly reported adverse events with eszopiclone were unpleasant taste and transient increases in RA symptoms. CONCLUSIONS: In this pilot study of patients with insomnia comorbid with RA, eszopiclone 3 mg improved all assessed sleep and daytime function measures over the treatment period, as well as some measures of RA-associated pain, disability, and quality of life. TRIAL REGISTRATION: clinicaltrials.gov Identifier: NCT00367965.
20706656 DAMPening inflammation by modulating TLR signalling. 2010 Damage-associated molecular patterns (DAMPs) include endogenous intracellular molecules released by activated or necrotic cells and extracellular matrix (ECM) molecules that are upregulated upon injury or degraded following tissue damage. DAMPs are vital danger signals that alert our immune system to tissue damage upon both infectious and sterile insult. DAMP activation of Toll-like receptors (TLRs) induces inflammatory gene expression to mediate tissue repair. However, DAMPs have also been implicated in diseases where excessive inflammation plays a key role in pathogenesis, including rheumatoid arthritis (RA), cancer, and atherosclerosis. TLR activation by DAMPs may initiate positive feedback loops where increasing tissue damage perpetuates pro-inflammatory responses leading to chronic inflammation. Here we explore the current knowledge about distinct signalling cascades resulting from self TLR activation. We also discuss the involvement of endogenous TLR activators in disease and highlight how specifically targeting DAMPs may yield therapies that do not globally suppress the immune system.